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Background/objectives: Ichthyosis refers to a group of genetic disorders characterized by extensive scaling of the skin. Syndromic ichthyosis, such as KID syndrome, is associated with mutations in connexin 26, resulting in a triad of keratosis, ichthyosis, and deafness. Cochlear implant (CI) is considered an effective rehabilitation option for severe hearing loss in these patients, though challenges related to skin complications may arise. This study aims to systematically review the existing literature to evaluate the effectiveness of CI in patients with ichthyosis, focusing on auditory and communicative abilities., Methods: A comprehensive literature search was conducted across PubMed, Scopus, and Web of Science databases according to the PRISMA statement. Studies were included based on the presence of genetically confirmed ichthyosis patients who underwent CI., Results: A total of 29 studies were identified, of which 11 met the inclusion criteria, encompassing 47 patients. Genetic analysis revealed GJB2 mutations in 40 patients, with a prevalence of the c.148G>A (D50N) mutation. All patients experienced sensorineural hearing loss, predominantly severe to profound. CI was performed in all patients, with significant improvements in speech discrimination and auditory thresholds (89.4%). Complications post-implant were noted in 78.6% of cases, primarily involving wound infections and dehiscence., Conclusions: Despite the potential for significant complications, the overall outcomes suggest that CI can markedly enhance the quality of life of subjects. Multidisciplinary approaches and careful surgical planning are crucial to managing these patients effectively. Future research should aim for larger sample sizes and extended follow-up periods to further understand CI outcomes in this population.

Recurrent respiratory papillomatosis (RRP) is a benign disease of the upper aerodigestive tract caused by human papillomavirus (HPV) types 6 and 11. The clinical course is unpredictable and some patients, especially younger children, experience a high rate of recurrence with a significant impact on their quality of life. The molecular mechanisms of HPV infection in keratinocytes have been extensively studied throughout the years, with particular regard to its role in causing malignant tumors, like cervical cancer and head and neck carcinomas. A minor but not negligible amount of the literature has investigated the molecular landscape of RRP patients, and some papers have studied the role of angiogenesis (the growth of blood vessels from pre-existing vasculature) in this disease. A central role in this process is played by vascular endothelial growth factor (VEGF), which activates different signaling cascades on multiple levels. The increased knowledge has led to the introduction of the VEGF inhibitor bevacizumab in recent years as an adjuvant treatment in some patients, with good results. This review summarizes the current evidence about the role of VEGF in the pathophysiology of RRP, the molecular pathways activated by binding with its receptors, and the current and future roles of anti-angiogenic treatment.

Subtotal petrosectomy (STP) is characterized by obliteration of the middle ear and occlusion of the external auditory canal. The advent of the endoscope has allowed a reduction in morbidity for some conditions such as cholesteatoma and other middle ear disorders, but STP still plays an important role. A retrospective review of medical records and videos of patients who had undergone STP was performed. Perioperative data and images were collected from various clinical cases who had undergone subtotal petrosectomy at our tertiary referral university hospital in Verona. We confronted our experience with a review of the literature to present the main indications for this type of procedure. STP allows a variety of diseases to be managed effectively as it offers the possibility of a definitive healing with radical clearance of temporal bone. Moreover, it can be safely combined with other procedures with a very low complication rate. Although the endoscope represents a revolution in ear surgery, STP, when indicated, is nowadays a surgical option that should be included in the otosurgeon's portfolio., Competing Interests: Conflict of interestAll the authors declare that they have no conflict of interest., (© Association of Otolaryngologists of India 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Background: Cochlear implantation (CI) in children with malformed ears can be challenging through the standard surgical technique. Several alternative approaches have been described. The endoscopic-assisted approach can be chosen as an effective and safe surgical technique, overcoming the drawbacks of the traditional approach., Material: We further describe a combined technique based on a limited mastoidectomy with no posterior tympanotomy and an endoscopic transmeatal approach to the round window (RW): the electrode is driven from the mastoid to the middle ear through the attic., Results: The concomitant endoscopic assistance allows for improved surgical vision, reducing the risk of major complications. The main advantages of this technique are related to better visualization of the RW for safe insertion of the electrode; avoidance of damage to the facial nerve (FN), due to direct visualization, and sparing the posterior tympanotomy; avoidance of subtotal petrosectomy, if not necessary., Conclusion: The purpose of this article, supported with a video file, is to describe step by step this endoscopic-assisted procedure in a patient with middle ear malformation., Competing Interests: Conflict of interest The authors have no conflict of interests, and the work was not supported or funded by any organization., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: To critically illustrate the personal experience with using the "Remote Check" application which remotely monitors the hearing rehabilitation level of cochlear implant users at home and further allows clinicians to schedule in-clinic sessions according to the patients' needs., Methods: 12-month prospective study. Eighty adult cochlear implant users (females n = 37, males n = 43; age range 20-77 years) with ≥ 36 months of cochlear implant experience and ≥ 12 months of stable auditory and speech recognition level volunteered for this 12-month long prospective study. For each patient, at the beginning of the study during the in-clinic session to assess the stable aided hearing thresholds and the cochlear implant integrity and patient's usage, the "Remote Check" assessment baseline values were obtained. "Remote Check" outcomes were collected at different times in the subsequent at-home sessions, to identify the patients that had to reach the Center. Chi-square test has been used for statistical analysis of the comparison of the "Remote Check" outcomes and in-clinic session results., Results: "Remote Check" application outcomes demonstrated minimal or no differences between all sessions. The at-home Remote Check application reached the same clinical outcomes as the in-clinic sessions in 79 out 80 of participants (99%) with high statistical significance (p < 0.05)., Conclusions: "Remote Check" application supported hearing monitoring in cochlear implant users that were not able to attend the in-clinic review during COVID-19 pandemic time. This study demonstrates that the application can be a useful routine tool also for clinical follow-up of cochlear implant users with stable aided hearing., (© 2023. The Author(s).)

Purpose: To describe the surgical procedure of exclusive endoscopic ossiculoplasty (EEO) with autologous grafts and evaluate audiological results, focusing on the advantages or drawbacks compared to the corresponding microscopic technique., Methods: A retrospective review of consecutive adult and pediatric patients affected by chronic otitis media (COM) with or without cholesteatoma who underwent EEO was conducted. Only autologous ossiculoplasty was included in the study. The procedure was performed by experienced surgeons of our institution between November 2014 and September 2019. Hearing outcomes were evaluated using postoperative air-bone gap (ABG) and success rates in different subgroups of patients and different types of ossiculoplasty (OPL) were analyzed. Our results were finally compared with the existing literature regarding both microscopic and endoscopic ossicular chain reconstruction., Results: In total, 74 endoscopic ossicular chain repair procedures performed within the study period met the inclusion criteria. Of these, 21 were pediatric patients (28%) and 53 were adults (72%). Surgical reconstruction procedures included 43 partial ossicular reconstructions (POR) and 31 total ossicular reconstructions (TOR). The postoperative ABG improved significantly compared to preoperative measurements, and the mean ABG closure was 7.85 dB HL (p = 0.00064). No statistically significant differences in audiological outcomes between TOR/POR techniques and pediatric/adult groups were found in our study cohort, with p values of 0.10 and 0.88, respectively., Conclusions: At present, EEO can be considered a valid surgical option for re-establishing a functioning ossicular chain with acceptable hearing restoration in children and adults. Further reports in wider case series are required to confirm these results., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Variations along the facial nerve (FN) course present considerable challenges in the surgical treatment of otosclerosis, often complicating the procedure. Existing knowledge of its tympanic tract and its implications primarily comes from microscopical procedures. This study aims to assess endoscopic findings of FN anatomy in a healthy tympanic cavity and its impact on the stapedotomy procedure, focusing on the risk of complications and functional hearing outcomes., Methods: A retrospective study on exclusive endoscopic stapedotoplasties between October 2014 and October 2021 at our Otorhinolaryngology University Department was carried out. An evaluation of intraoperative endoscopic findings reviewed in surgical descriptive and/or video records was conducted to assess their potential negative impact on the surgery. Demographic data, preoperative and postoperative hearing thresholds, as well as intraoperative and postoperative complications were analyzed., Results: One hundred fifty-seven subjects were included. A FN partially overhanging the oval window was observed in 7.3% (n=12): 10 prolapsing with bony canal dehiscence and 2 without any detected dehiscence. Each procedure was successfully completed without any issues related to the anomalous anatomy, and in no case, switching to the microscope for the handling of the prosthesis near the dehiscent nerve was required. No facial paralysis occurred, with an early- or long-term postoperative House-Brackman grade of 1 (n=157, 100%). Only 3/157 patients (1.9%) showed a sensorineural threshold reduction of ≥20 dB HL, but a significant air-bone gap improvement was observed (mean closure of 18.36 dB HL, P -lt; .0001)., Conclusion: The endoscope promotes a concrete description of tympanic FN anatomy, and endoscopic stapes surgery appears to be a safe and viable option when dehiscent or prolapsed FNs reduce the footplate's exposure.

Laryngeal cancer is a very common tumor in the upper aero-digestive tract. Understanding its biological mechanisms has garnered significant interest in recent years. The development of laryngeal squamous cell carcinoma (LSCC) follows a multistep process starting from precursor lesions in the epithelium. Various risk factors have been associated with laryngeal tumors, including smoking, alcohol consumption, opium use, as well as infections with HPV and EBV viruses, among others. Cancer development involves multiple steps, and genetic alterations play a crucial role. Tumor suppressor genes can be inactivated, and proto-oncogenes may become activated through mechanisms like deletions, point mutations, promoter methylation, and gene amplification. Epigenetic modifications, driven by miRNAs, have been proven to contribute to LSCC development. Despite advances in molecular medicine, there are still aspects of laryngeal cancer that remain poorly understood, and the underlying biological mechanisms have not been fully elucidated. In this narrative review, we examined the literature to analyze and summarize the main steps of carcinogenesis and the risk factors associated with laryngeal cancer.

Background: The Cochlear™ Osia ® 2 System is an active transcutaneous bone-anchored hearing implant with a newly developed piezoelectric transducer that is fixed to a titanium implant (BI300)., Methods: It uses digital piezoelectric stimulation to bypass non-functional areas of the natural hearing system and send sound directly to the cochlea. This device is designed to meet the needs of patients with unilateral and bilateral conductive or mixed hearing loss and single-sided deafness., Conclusion: We show step by step how to place the new active transcutaneous bone conduction implant, Cochlear™ Osia ® 2 System, which utilizes a piezoelectric actuator anchored to the mastoid bone through an osseointegrated screw., (© 2022. The Author(s).)

Primary tumors of the airways in the pediatric population are very rare entities. For this reason, little is known about the pathogenesis of these neoplasms. Understanding the biology has different practical implications: for example, it could help in the differential diagnosis, have a prognostic significance, or may lead to the development of a targeted therapy. The aim of this article is to present the current knowledge about pediatric airways tumors, focusing on the molecular mechanisms that cause the onset and progression of these neoplasms. After a brief introduction of epidemiology and clinical presentation, the tumorigenesis of the most frequent pediatric airways tumors will be described: Juvenile-onset recurrent respiratory papillomatosis (JORRP), Subglottic Hemangiona (SH), Rhabdomyosarcoma (RMS), and Mucoepidermoid carcinoma (MEC).

Background : Cochlear implantation (CI) following endoscopic transcanal infrapromontorial vestibular schwannoma (VS) dissection is a feasible intervention in intracanalicular VS, with minimal extension into the cerebellopontine angle, but no audiologic results have ever been reported in the literature. Methods: From 2015 to 2021 in the Otorhynolaryngology Departments of Modena and Verona, three patients underwent this intervention. All were suffering from sporadic left-sided intracanalicular Koos I VS. Intraoperative electrically evoked auditory brainstem responses and electrophysiological measurements were performed before and after the placement of the electrode array, respectively. Since device activation one month after the surgery, each patient was followed up with audiometric tests, data logging, electrode impedance measurements and neural response telemetry performed at each scheduled fitting session at 15 days and 3, 6, 12 and 24 months. Results: Only in patient No. 3, an auditory benefit was observed and still evident even 36 months after activation. Impedances increased progressively in patient No. 1 and a benefit was never reported. Patient No. 2 left the follow-up for worsening comorbidities. Conclusions: CI following transcanal infrapromontorial VS resection is a beneficial intervention. The residual cochlear nerve after the tumour dissection and the course of electrophysiological measurements in the postoperative period were the main predictive factors for audiological outcomes.

Purpose: The aim of this study is to evaluate speech perception outcomes after a frequency reallocation performed through the creation of an anatomically based map obtained with Otoplan ® , a tablet-based software that allows the cochlear duct length to be calculated starting from CT images., Methods: Ten postlingually deafened patients who underwent cochlear implantation with MED-EL company devices from 2015 to 2019 in the Tertiary referral center University Hospital of Verona have been included in a retrospective study. The postoperative CT scans were evaluated with Otoplan ® ; the position of the intracochlear electrodes was obtained, an anatomical mapping was carried out and then it was submitted to the patients. All patients underwent pure tonal and speech audiometry before and after the reallocation and the audiological results were processed considering the Speech Recognition Threshold (SRT), the Speech Awareness Threshold (SAT) and the Pure Tone Average (PTA). The differences in the PTA, SAT and SRT values before and after the reallocation were determined. The results were statistically processed using the software Stata with a significance value of α < 0.05., Results: The mean values of SRT (61.25 dB versus 51.25 dB) and SAT (49 dB versus 41 dB) were significantly lower (p: 0.02 and p: 0.04, respectively) after the reallocation. No significant difference was found between PTA values (41.5 dB versus 39.25 dB; p: 0.18)., Conclusions: Our preliminary results demonstrate better speech discrimination and rapid adaptation in implanted postlingually deaf patients after anatomic mapping and subsequent frequency reallocation., (© 2022. The Author(s).)